Overview
Lichen myxedematosus, also known as papular mucinosis, is a rare skin condition in which a substance called mucin builds up in the skin. This causes small, firm, waxy bumps (papules) to appear, most often on the hands, arms, face, neck, and trunk. The skin may also become thickened and hardened in affected areas. There are two main forms of this disease: a generalized form called scleromyxedema, which is more serious and can affect internal organs, and a localized form that mainly affects the skin. Scleromyxedema is often linked to an abnormal protein in the blood called a paraprotein (usually an IgG lambda type), which is produced by certain immune cells. The localized forms tend to be milder and may not have this blood abnormality. Symptoms vary depending on the form. In the localized type, patients may notice clusters of small bumps in specific areas of the body. In scleromyxedema, the skin changes are more widespread, and patients can experience stiffness, difficulty moving their joints, and problems with swallowing or breathing if internal organs are involved. Neurological symptoms such as confusion, seizures, or coma can occur in severe cases. The exact cause of lichen myxedematosus is not fully understood, but it is not considered a hereditary (inherited) condition. Treatment depends on the severity. Localized forms may not need aggressive treatment, while scleromyxedema often requires systemic therapies such as intravenous immunoglobulin (IVIG), which is currently considered a first-line treatment. Other options include melphalan, thalidomide, and autologous stem cell transplant in refractory cases.
Key symptoms:
Small, firm, waxy skin bumps (papules)Thickened or hardened skinSkin stiffnessDifficulty moving joints due to tight skinSwelling of the face or handsDifficulty swallowingMuscle weaknessNumbness or tingling in hands and feetShortness of breathConfusion or changes in mental statusSeizuresWeight lossFatigueSkin that looks shiny or waxy
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Lichen myxedematosus.
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Specialists
View all specialists →No specialists are currently listed for Lichen myxedematosus.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Lichen myxedematosus.
Community
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Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Which form of lichen myxedematosus do I have — localized or scleromyxedema?,Do my blood tests show a paraprotein, and what does that mean for my health?,What treatment do you recommend, and how long will I need it?,What are the signs of serious complications I should watch for at home?,How often will I need follow-up visits and blood tests?,Are there any clinical trials or newer treatments available for this condition?,Should I see any other specialists, such as a hematologist or neurologist?
Common questions about Lichen myxedematosus
What is Lichen myxedematosus?
Lichen myxedematosus, also known as papular mucinosis, is a rare skin condition in which a substance called mucin builds up in the skin. This causes small, firm, waxy bumps (papules) to appear, most often on the hands, arms, face, neck, and trunk. The skin may also become thickened and hardened in affected areas. There are two main forms of this disease: a generalized form called scleromyxedema, which is more serious and can affect internal organs, and a localized form that mainly affects the skin. Scleromyxedema is often linked to an abnormal protein in the blood called a paraprotein (usually
How is Lichen myxedematosus inherited?
Lichen myxedematosus follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Lichen myxedematosus typically begin?
Typical onset of Lichen myxedematosus is adult. Age of onset can vary across affected individuals.